Analysis Tools
behavior/neurological
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• mice show continuous running around the cage when the cage is moved, indicating a preconvulsant behavior
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• mice exhibit increased susceptibility to pentylenetetrazol-induced seizures
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• mice are hesitant to investigate surroundings when placed in a new environment
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• abnormal whisker twitch reflex
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• all mice at 6 months of age, and fewer than half at 3 months, exhibit abnormal hindlimb clasping reflex
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• mice begin to show ataxic gait at 6 months of age, that involves awkward movements of the hindlimbs
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• greater variability in the width of steps
• however, no differences are seen on fixed-speed or accelerated rotarod
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• in the open field, mice travel a lesser distance at a slower speed than wild-type mice
• mice spend less time traveling at a speed greater than 50 mm/sec and exhibit fewer crossings
• however, no differences are seen in the amount of time spend in the center versus the outside of the arena
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nervous system
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• mice exhibit increased susceptibility to pentylenetetrazol-induced seizures
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• RNA aggregates containing expanded AUUCU repeats are seen in the brain of 3 and 6 month old mice
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• frontal lobe contains neurons with large vacuoles
• vacuoles increase in number and magnitude with increasing age such that brains at 3 months of age contain small vacuoles in about 20% of neurons, while brains at 18 months contain large vacuoles in nearly 100% of neurons
• vacuoles contain glycogen
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• loss of neurons in the CA3 region of the hippocampus, in the pontine nuclei, and in the cerebral cortex, but not in the cerebellum, at 6 months of age, with many atrophic neurons showing small perikarya, corkscrew-like dendrites, and dense basophilic nuclei
• however, no evidence of apoptosis or gliosis is seen in the brain
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• loss of neurons in the CA3 region of the hippocampus at 6 months of age
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| spinocerebellar ataxia type 10 | DOID:0050960 |
OMIM:603516 |
J:222081 | |
